Reticulohistiocytoma of the dorsum (Crosti's disease) and other B-cell lymphomas.
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Biomedical subjects
Publications and source records attributed to E Alessi.
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Oral hairy leukoplakia was first described in homosexual men infected with the human immunodeficiency virus. It is thought to be caused by infection with both the Epstein-Barr virus and human papillomavirus. We report 59 cases of oral hairy leukoplakia. The disease was diagnosed in patients in all risk groups and was categorized in all classes of the Walter Reed classification without significant differences in prevalence. Epstein-Barr virus could be demonstrated in all tissue samples examined; human papillomavirus was found in only a few specimens. In our series oral hairy leukoplakia had a chronic course, although temporary spontaneous healing occurred in some cases. Its appearance was a poor prognostic sign because acquired immunodeficiency syndrome developed in a significant proportion of patients within a few months of onset.
Pityriasis lichenoides is usually classified into an acute and a chronic form. From a review of 89 cases of the disease seen since 1974 it seems that a more realistic classification into three main groups, according to the distribution of pityriasis lichenoides lesions, could be made, namely, a diffuse, a central, and a peripheral form, each characterized by a different clinical course. Conversely, no correlations were detected in our series between the severity of skin lesions and their distribution or the overall course of the disease. None of our cases suggests the possible evolution of pityriasis lichenoides into lymphomatoid papulosis. Although no infectious causative agent has been identified, a viral origin seems likely in some cases. Most patients responded favorably to UVB irradiation. Our conclusions are (1) that pityriasis lichenoides is probably a clinical disorder with a diverse etiology and (2) that its classification by distribution seems more useful than its subdivision into an acute and a chronic form.
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The Italian Cooperative Group on AIDS-related tumors has collected 435 cases of HIV-associated tumors since December 1986. The following conclusions can be drawn from this IVDA-based series: (1) at least 15% of AIDS cases are associated with tumors; (2) the number of malignant lymphomas (high-grade non-Hodgkin's lymphoma [NHL], Hodgkin's disease [HD] is comparable to that of Kaposi's sarcoma (KS) (188 vs. 198); (3) KS among AIDS patients is less common than in countries where homosexual men are the main group affected by AIDS. However, KS also affects intravenous drug abusers (IVDA) almost exclusively males, with characteristics similar to those observed among homosexual men; (4) HD is associated with an aggressive course; (5) anal and oral primary tumors as well as oral and anal involvement of NHL are very rare; (6) testicular cancers occur in patients mainly with early HIV infection, without adversely affecting the dosage of radiotherapy and chemotherapy; (7) cervical cancer successfully treated with conization suggests that PAP test screening in young IVDA women is warranted; (8) lung cancer occurs in a young age group with rapid progression and death.
Fourteen cases of primary cutaneous B-cell lymphomas were investigated at the immunohistochemical and molecular level to further characterize this newly defined entity. Neoplastic cells from all cases, phenotyped with a panel of monoclonal antibodies, were positive for HLA-DR, for the B-cell markers CD19, CD22, but not CD23 (except one case), and negative for the T-cell marker CD2. Monoclonal immunoglobulin light chains were demonstrated in six cases. The reactivity with the Ki-67 monoclonal antibody indicated that the neoplastic cells are proliferating. In five biopsies the presence of dendritic cells infiltrating the neoplastic areas was revealed using the monoclonal antibody Kim4b. By Southern blot analysis, clonal rearrangement of the immunoglobulin heavy chain gene (involving one or both alleles) was shown in 12 of 14 cases and of the light chain genes in 13 cases. The bcl-2 oncogene, normally involved in nodal follicular lymphomas, was in germ-line configuration. The c-myc and the beta and gamma chain genes of the T-cell receptor were also in the germ-line configuration. None of the cases presented Epstein-Barr virus sequences. These data indicate that primary cutaneous lymphomas of B-cell origin share morphological and phenotypic similarities with the nodal B-cell lymphomas of follicular histotype, are proliferating, and express in 45% of cases clear monoclonal immunoglobulin light chain; the molecular analysis confirms the B-cell derivation and the monoclonal nature of this neoplasia; it also shows that neither bcl-2 nor c-myc oncogenes are involved and that no inappropriate rearrangements of the T-cell receptor genes are found in this lymphoma.
Anaplastic large cell lymphoma is a subtype of nodal and extranodal lymphoproliferative disorder characterized by peculiar histopathological features and the positivity of lymphoid proliferating cells for Ki-1/Ber-H2 monoclonal antibody. A case, misdiagnosed as cutaneous metastasis and treated by surgery, in a woman with a history of previous neoplasms, is reported. In spite of the histopathological malignancy of the tumor, the patient is alive and free from recurrent or metastatic disease after 3 years of follow-up.
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To clarify the nature of reticulohistiocytoma of the dorsum, 19 cases, including three of the seven original cases described by Crosti, were evaluated clinically, histologically, and immunologically. In seven cases gene rearrangement analysis was also performed. Results indicate that reticulohistiocytoma of the dorsum must be considered a primary cutaneous B cell lymphoma of follicular center cell origin. This localized skin disease has a very slowly progressive course, with many patients showing no systemic involvement even after prolonged follow-up.
The mucocutaneous manifestations observed in 516 patients infected with human immunodeficiency virus attending an outpatient clinic for acquired immunodeficiency syndrome in Milan, Italy, from July 1985 to March 1987 are listed, and the clinicopathologic aspects of these disorders are reported. The prognostic significance of some human immunodeficiency virus-associated dermatoses is also discussed.
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We treated a 13-year-old girl who had annular erythematous lesions with central atrophic areas, which had been present on her trunk and limbs for 4 months. Histological examination revealed patchy dermal lymphohistocytic infiltration with multinucleated giant cells which were phagocytosing elastic fibers, causing them to disappear. The active border of the lesions regressed after intradermal injection of corticosteroids. The classification of the disease and its differential diagnosis from the usual granuloma annulare, inflammatory anetoderma, O'Brien's actinic granuloma, and Convit's disease are discussed.
A homosexual male, seropositive to HIV and with previously documented syphilitic infections, developed a slowly growing nodule on his left wrist (possibly a chancre) followed, after 2 months, by a few scattered, large papular lesions. Serological evidence of active syphilis was obtained and treponema were identified in the initial lesion by means of immunofluorescent staining. Treatment with both aqueous crystalline penicillin G and benzathine penicillin G healed the lesions. This represents a further case of an atypical presentation of early syphilis in an immunocompromised host.
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